Sathe Rujuta, Shrestha Gyaneshwar, Terango Aria, Tabibzadeh David, Rajaraman Rajsekar R, Nariai Hiroki, Hussain Shaun A
Division of Pediatric Neurology, University of California, Los Angeles, California, USA.
UCLA Mattel Children's Hospital, Los Angeles, California, USA.
Epilepsia Open. 2025 Feb;10(1):314-320. doi: 10.1002/epi4.13099. Epub 2024 Nov 21.
Vigabatrin-associated brain abnormalities on MRI (VABAM) are observed in approximately 20% of children who receive vigabatrin for treatment of infantile epileptic spasms syndrome. Although usually reversible and asymptomatic, VABAM is occasionally symptomatic. Whereas asymptomatic VABAM appears to be dose-dependent, symptomatic VABAM is possibly associated with co-administration of vigabatrin and hormonal therapy (i.e., corticosteroids or adrenocorticotropic hormone). With retrospective study of a cohort of vigabatrin-treated children, we evaluated candidate risk factors for VABAM. Among 108 children with detailed vigabatrin exposure data, we identified VABAM in 17 children (11 symptomatic). Symptomatic VABAM was strongly associated with simultaneous exposure to hormonal therapy (p = 0.001). Neither symptomatic nor asymptomatic VABAM were associated with peak vigabatrin dose. Although these data support the hypothesis that symptomatic VABAM risk is higher with coadministration of vigabatrin and hormonal therapy, this study does not establish a causal link. Further study is warranted to better understand the pathogenesis of VABAM and devise strategies to mitigate risk. Clinicians should carefully weigh the potential risk of symptomatic vigabatrin toxicity against the known benefit of vigabatrin and hormonal therapy coadministration. PLAIN LANGUAGE SUMMARY: Several case reports suggest that the combination of vigabatrin and hormonal therapy for treatment of infantile spasms may provoke an adverse reaction known as symptomatic vigabatrin MRI toxicity (sVABAM, which includes characteristic changes on MRI images and associated symptoms). In response to these reports, we studied a large single-center cohort of children with infantile spasms and determined that combination therapy is indeed statistically associated with sVABAM. However, we have not proven that combination therapy actually causes sVABAM. Further study is needed to clarify the nature of sVABAM and risk factors thereof.
在接受vigabatrin治疗婴儿痉挛综合征的儿童中,约20%会出现MRI上与vigabatrin相关的脑异常(VABAM)。虽然VABAM通常是可逆且无症状的,但偶尔也会出现症状。无症状的VABAM似乎与剂量有关,而有症状的VABAM可能与vigabatrin和激素疗法(即皮质类固醇或促肾上腺皮质激素)的联合使用有关。通过对一组接受vigabatrin治疗的儿童进行回顾性研究,我们评估了VABAM的候选风险因素。在108名有详细vigabatrin暴露数据的儿童中,我们在17名儿童中发现了VABAM(11名有症状)。有症状的VABAM与同时接受激素治疗密切相关(p = 0.001)。有症状或无症状的VABAM均与vigabatrin峰值剂量无关。虽然这些数据支持vigabatrin与激素疗法联合使用时出现有症状VABAM风险更高的假设,但本研究并未确立因果关系。有必要进一步研究以更好地了解VABAM的发病机制并制定降低风险的策略。临床医生应仔细权衡有症状的vigabatrin毒性的潜在风险与vigabatrin和激素疗法联合使用的已知益处。
几例病例报告表明,vigabatrin与激素疗法联合用于治疗婴儿痉挛可能会引发一种称为有症状vigabatrin MRI毒性的不良反应(sVABAM,包括MRI图像上的特征性变化及相关症状)。针对这些报告,我们研究了一个大型单中心队列的婴儿痉挛患儿,确定联合治疗确实与sVABAM在统计学上相关。然而,我们尚未证明联合治疗实际上会导致sVABAM。需要进一步研究以阐明sVABAM的性质及其风险因素。